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Hearing Program

The fields of cellular and molecular biology have furthered hearing research. A multitude of genes for syndromic and nonsyndromic forms of hearing impairment including autosomal dominant and recessive, X-linked and mitochondrial modes of transmission have been located in specific regions of the human genome. In addition, clinically relevant genes essential for normal auditory development and/or function are being identified and cloned at a rapid pace.

Other cochlear-specific genes have been isolated from enriched membranous labyrinth cDNA libraries. New technology, including the development of detailed maps of expressed sequence tags (EST) coupled with the use of inner ear specific cDNA libraries, exon trapping and cDNA library enrichment procedures, have facilitated gene cloning. Once cloned, the molecular biology of hearing and the role of particular proteins in the development and/or maintenance of the inner ear can be determined. Mouse models of hereditary hearing impairment have been instrumental in mapping and cloning many deafness genes. Because of the utility of the mouse for such studies, additional mouse models of deafness are being created through mutagenesis and screening programs as well as targeted mutation of deafness genes found in man. In addition, mouse models are being used to study the function of the proteins encoded by deafness genes and to test therapeutic approaches. These advances offer researchers many opportunities to study the characteristics of deafness, hereditary factors involved in hearing loss, and genes that are critical for the development and maintenance of the human ear. Great strides are being made in the study of properties of auditory sensory cells, and of characteristics of the response of the inner ear to sound.

Scientific advances have also been translated into cochlear implants, digital hearing aids, and tactile devices that provide information by stimulating the skin. Great strides are being made in the study of properties of auditory sensory cells, and of characteristics of the response of the inner ear to sound. Research has verified that despite substantial variability in the performance of children who have received cochlear implants, most demonstrate marked improvements in speech perception and production. Speech produced by children who use multichannel cochlear implants is usually more accurate than the speech produced by children with comparable hearing impairment using vibrotactile devices or hearing aids. Cochlear implants also positively influence children's receptive and expressive language skills. The longer children use their implants, the greater their language ability.

To achieve the most benefit from their implants, however, children generally need extensive oral-auditory training following implantation and also benefit from periodic audiological assessments. Cochlear implants have benefited children who are congenitally deaf as well as those who are postlingually deaf. The vast majority of adult implant recipients derive substantial benefit in conjunction with speechreadng, and many can communicate effectively without speechreading and are able to communicate by telephone. Dedication to research on cochlear implants throughout the world will improve the capabilities of current implant users and improve our understanding of the auditory system.

New insights have been gained concerning the encoding of complex signals transmitted from the auditory nerve to the brain. The relationship between the neural codes for sound intensity, frequency, duration and temporal characteristics of auditory signals and the perception of the stimulus variables has been further clarified. Valuable progress has been made in understanding the structure and function of efferent feedback pathways to the inner and middle ear. There is now good evidence that this system may aid in the detection of signals in noisy environments and serve to protect the ear from acoustic injury.

Gains have been made about the ways in which the brain creates maps of auditory space and how the maps interact with visual space. This research may have implications in treatment of children who acquire hearing loss in infancy or early childhood. Further, psychoacoustic and electrophysiologic studies of infants and children are providing important new insights into the development of functional hearing.

In the aging auditory system, discoveries have been made demonstrating changes in the regulation of fluid composition and autoregulation of cochlear blood flow which may underlie some of the biologic effects of aging on auditory function. Improved behavioral and electrophysiological techniques for measuring auditory function are providing more accurate assessments of the peripheral and central components of age-related hearing impairment.

Recent development of animal models for bacterial and viral infections hold promise for new diagnostic and therapeutic approaches to sensorineural hearing loss caused by infections. Antiviral drugs may find rapid application in the treatment for these conditions with the advent of suitable animal models in which to test efficacy. In addition, models will allow a greater understanding of why and to what degree infants and children are susceptible to ototoxic drugs used in the treatment of infections.

Otitis media continues to be a significant focus of research because of its prevalence and cost to society. Important risk factors have been identified. Studies of the eustachian tubes have provided new information on tubal mechanics, surfactant-like (fluid) substances and middle ear pressure regulation. The role of bacterial biofilms in chronic otitis media is a new and promising area of investigation. State-of-the-art molecular, genetic and genomic techniques are being used to identify genes that may predispose an individual to chronic otitis media. These techniques are also being used to define the specific molecular changes that allow viral and bacterial infection of the middle ear as well as the host/pathogen interactions that facilitate the disease process.

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